Causes of Pulmonary Hypertension
World Health Organization Classification

Pulmonary Arterial Hypertension

• Idiopathic pulmonary arterial hypertension
• Familial pulmonary arterial hypertension
• Scleroderma, Systemic Lupus Erythematosus (SLE)
• Intra-cardiac shunts (ASD, VSD etc)
• Porto-pulmonary hypertension (liver disease)
• Drugs (Phen-Fen, cocaine and others)
• HIV
• Sickle cell disease and certain other blood diseases

Pulmonary Hypertension Due to Lung Disease

• Pulmonary Fibrosis
• Emphysema
• Sleep Apnea

Pulmonary Hypertension Due to Heart Disease

• Heart failure
• Mitral valve disease
• Coronary artery disease
• Cardiomyopathies

Pulmonary Hypertension Due to Embolic Disease

• Blood clots that fail to resolve with treatment

Pulmonary Hypertension Due to Miscellaneous Causes

• Sarcoidosis and others
  

Tests and Medications

Echocardiogram. Sometimes called an “echo”. This is a sound wave (or ultrasound) test of the heart. It’s the same kid of technology that’s used to “see” babies before they are born. The test takes about 30-45 minutes. It requires no preparation, sedation, or recovery time. An “echo” creates a moving picture of the four heart chambers and the four heart valves. A physician can use the pictures to evaluate the function of the pumping chambers and valves. Pulmonary artery pressure can be estimated with this test.

Electrocardiogram. Sometimes called a heart tracing. Electrodes are placed on the chest arms, and legs and record the heart’s electrical activity. The tracing can provide evidence of damage, enlargement or abnormal heart rhythms.

Right heart catheterization. A catheter (plastic tube) is inserted into a vein in the groin and threaded up the vein to the heart and into the pulmonary artery where the pressure can be accurately measured. The heart’s ability to pump blood can also be measured. Tests can be made that determine how well the pulmonary arteries respond to medication.

Catheterization is an “out-patient” procedure that lasts one to two hours, but additional preparation time and monitoring after the test will use up a full day. If you are taking “blood thinners”, these will be stopped several days before the test. You will be instructed about what you may eat prior to the test. Someone will have to drive you home after the test.

6 minute walk. You will be asked to walk on level ground as quickly as possible for 6 minutes. Your oxygen levels will be measured before and after the walk. This test estimates your physical capacity. This test is usually performed in the clinic and requires no preparation.

Pulmonary function testing (PFT). These are breathing tests. You will be asked to blow into a tube as hard and as long as you possibly can. The test will be repeated several times. Technicians will encourage you with each test. This outpatient test requires about one hour to complete.

Chest CT (computerized tomographic) scan. This x-ray test evaluates the blood vessels in the lung for blood clots. It requires an IV in the arm and an injection of “contrast” material during the test. The testing session generally lasts 30-45 minutes. It is an “out patient” procedure and requires no sedation, but may require pre-medication if you have a “contrast” allergy, or an allergy to shell fish.

Sleep study. If your doctor believes that your pulmonary hypertension is associated with breathing difficulties during sleep, you will need a “polysomnogram” (sleep study). This test is performed overnight in a special laboratory. Your brain waves, heart rate, breathing and oxygen levels will be measured overnight using small electrodes attached to your body.

Warfarin. An anti-coagulant (blood thinner) drug taken by mouth once a day. Warfarin prevents clots from forming inside the small blood vessels in the lung. Studies of patients with pulmonary hypertension show that patients who take this drug are more likely to survive longer. A blood test called the “pro-time” must be obtained on a regular basis to monitor warfarin’s effect. The test results are reported as the INR and the goal INR value is usually between 2.0 and 2.5. Adjustments to the dose are made on the basis of the test. Bruising and bleeding are the side-effects of warfarin and are related to INR values that are too high. Many medications and some foods can alter the INR. You should carry a card or wear a bracelet stating that you are taking warfarin.

Calcium channel blockers. Vasodilator and anti-hypertensive drugs taken by mouth once or twice a day. This class of drugs is relatively inexpensive, but usually requires high doses that my cause side effects such as edema (swollen ankles), dizziness, weakness, nausea ,low blood pressure and slow heart rate. Patients who have good responses to calcium channel blockers have a very good prognosis.

Epoprostenol (Flolan ®). A vasodilator administered by continuous infusion through a catheter implanted in a large vein. This drug has been shown to improve symptoms, walking distance and survival in patients with pulmonary arterial hypertension. Most patients can benefit from this drug, but it is not a simple drug to use. Patients must be willing to learn about the drug and its pump delivery system. Each patient (and a partner) must be individually trained to prepare the drug using sterile technique, to adjust the pump, and be prepared to handle problems. Side effects of the drug itself include jaw pain, skin flushing and diarrhea. Patients needing this drug are generally admitted to the hospital for 3-5 days. The catheter is inserted on the first day, the medication is started and intensive training is continued. This drug can only be obtained through a specialty pharmacy after your doctor submits a special application for you.

“Out of Breath” : A short “comic book” explanation of pulmonary hypertension and prostacyclin. http://www.unither.com/comicbook/)

Treprostinil (Remodulin ®). A vasodilator that can be administered in two ways:
1) by continuous infusion into a vein just like Epoprostenol, or
2) by continuous infusion through a needle just under the skin.

This drug is similar to Epoprostenol, but offers several advantages. The medication does not have to be mixed; nor does it have to be chilled. When it is administered under the skin, a smaller pump can be used. Treprostinil causes the same side effects as Epoprostenol and when it is given under the skin, it causes significant irritation and pain at the skin site. This drug can only be obtained through a specialty pharmacy after your doctor submits a special application for you.

Bosentan (Tracleer ®). A vasodilator taken by mouth twice a day. This drug has been shown to improve symptoms, and walking distance in patients with pulmonary arterial hypertension. It also delays the onset of complications of pulmonary hypertension. Bosentan’s major side-effect is liver injury. Monthly blood tests must be performed to measure liver function and to make adjustments to the dose if necessary. This drug can only be obtained through a specialty pharmacy after your doctor submits a special application for you. http://www.tracleer.com

Sildenafil (Revatio ®). A vasodilator taken by mouth three times a day. This drug has been shown to improve symptoms, and walking distance in patients with pulmonary arterial hypertension. Sildenafil’s major side effects are headache, upset stomach, diarrhea and stuffy nose.

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